Cystic fibrosis, The Lancet, vol.361, issue.9358, pp.681-689, 2003. ,
DOI : 10.1016/S0140-6736(03)12567-6
URL : https://hal.archives-ouvertes.fr/hal-01361411
CFTR as a cAMP-dependent regulator of sodium channels, Science, vol.261, issue.2, pp.847-850, 1995. ,
DOI : 10.1089/hum.1992.3.3-253
CFTR and chaperones, Journal of Molecular Neuroscience, vol.2323, pp.41-481, 2004. ,
DOI : 10.1385/jmn:23:1-2:041
Correction of the ??Phe508 Cystic Fibrosis Transmembrane Conductance Regulator Trafficking Defect by the Bioavailable Compound Glafenine, Molecular Pharmacology, vol.77, issue.6, pp.922-930, 2010. ,
DOI : 10.1124/mol.109.062679
Rescue of ?F508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules, AJP: Lung Cellular and Molecular Physiology, vol.290, issue.6, pp.1117-1130, 2005. ,
DOI : 10.1152/ajplung.00169.2005
Disruption of cytokeratin-8 interaction with F508del-CFTR corrects its functional defect, Human Molecular Genetics, vol.21, issue.3, 2011. ,
DOI : 10.1093/hmg/ddr496
Trimethylangelicin reduces IL-8 transcription and potentiates CFTR function, AJP: Lung Cellular and Molecular Physiology, vol.300, issue.3, pp.380-390, 2010. ,
DOI : 10.1152/ajplung.00129.2010
Pathophysiology of gene-targeted mouse models for cystic fibrosis, Physiological Reviews, vol.79, p.193, 1999. ,
Phenotypic characterisation of patients with intermediate sweat chloride values: towards validation of the European diagnostic algorithm for cystic fibrosis, Thorax, vol.64, issue.8, pp.683-691, 2009. ,
DOI : 10.1136/thx.2008.104752
Ataluren (PTC124) Induces Cystic Fibrosis Transmembrane Conductance Regulator Protein Expression and Activity in Children with Nonsense Mutation Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine, vol.182, issue.10, 2010. ,
DOI : 10.1164/rccm.201001-0137OC
Mutation, New England Journal of Medicine, vol.363, issue.21, pp.1991-2003, 2010. ,
DOI : 10.1056/NEJMoa0909825
Partial Correction of Cystic Fibrosis Defects with PLGA Nanoparticles Encapsulating Curcumin, Molecular Pharmaceutics, vol.7, issue.1, pp.86-93, 2010. ,
DOI : 10.1021/mp900138a
Airway Delivery of Low-Dose Miglustat Normalizes Nasal Potential Difference in F508del Cystic Fibrosis Mice, American Journal of Respiratory and Critical Care Medicine, vol.179, issue.11, pp.1022-1028, 2009. ,
DOI : 10.1164/rccm.200901-0049OC
Ion Transport across Cystic Fibrosis Nasal Epithelium, American Journal of Respiratory and Critical Care Medicine, vol.171, issue.8, pp.868-871, 2005. ,
DOI : 10.1164/rccm.200311-1508OC
Examining basal chloride transport using the nasal potential difference response in a murine model, Am J Physiol Lung Cell Mol Physiol, vol.281, pp.1173-1179, 2001. ,
Ion transport across CF and normal murine olfactory and ciliated epithelium, AJP: Cell Physiology, vol.296, issue.6, p.1301, 2009. ,
DOI : 10.1152/ajpcell.00578.2008
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2692423
Preclinical Evidence that Sildenafil and Vardenafil Activate Chloride Transport in Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine, vol.177, issue.5, pp.506-515, 2008. ,
DOI : 10.1164/rccm.200703-344OC
Validation of Nasal Potential Difference Measurements in Gut-Corrected CF Knockout Mice, American Journal of Respiratory Cell and Molecular Biology, vol.39, issue.4, pp.490-496, 2008. ,
DOI : 10.1165/rcmb.2007-0385OC
CFTR involvement in nasal potential differences in mice and pigs studied using a thiazolidinone CFTR inhibitor, AJP: Lung Cellular and Molecular Physiology, vol.287, issue.5, p.936, 2004. ,
DOI : 10.1152/ajplung.00354.2003
Airway gene transfer in mouse nasal-airways: importance of identification of epithelial type for assessment of gene transfer, Gene Therapy, vol.7, issue.21, pp.1810-1815, 2000. ,
DOI : 10.1038/sj.gt.3301317
Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis, Thorax, vol.65, issue.6, pp.539-544, 2010. ,
DOI : 10.1136/thx.2009.123422
Statistical methods for assessing agreement between two methods of clinical measurement, International Journal of Nursing Studies, vol.47, issue.8, pp.307-310, 1986. ,
DOI : 10.1016/j.ijnurstu.2009.10.001
Direct Comparison of NPPB and DPC as Probes of CFTR Expressed in Xenopus Oocytes, Journal of Membrane Biology, vol.175, issue.1, pp.35-52, 2000. ,
DOI : 10.1007/s002320001053
Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator, Proceedings of the National Academy of Sciences, vol.92, issue.15, pp.2674-2679, 1998. ,
DOI : 10.1073/pnas.92.15.6832
Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents, American Journal of Physiology -Cell Physiology, vol.266, pp.1464-1477, 1994. ,
Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation, Proceedings of the National Academy of Sciences, vol.27, issue.1, pp.2965-2970, 2006. ,
DOI : 10.1016/S0301-472X(99)00032-6
Defective respiratory amiloride-sensitive sodium transport predisposes to pulmonary oedema and delays its resolution in mice, The Journal of Physiology, vol.271, issue.3, pp.857-865, 2004. ,
DOI : 10.1111/j.1469-7793.2000.00771.x
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1665279
SERCA Pump Inhibitors Do Not Correct Biosynthetic Arrest of ?F508 CFTR in Cystic Fibrosis, American Journal of Respiratory Cell and Molecular Biology, vol.34, issue.3, pp.355-363, 2006. ,
DOI : 10.1165/rcmb.2005-0286OC
Measurement of airway ion transport assists the diagnosis of cystic fibrosis, Pediatric Pulmonology, vol.2, issue.8, pp.789-795, 2010. ,
DOI : 10.1136/bmj.313.7059.744
The Use and Misuse of the Coefficient of Variation in Organizational Demography Research, Sociological Methods & Research, vol.30, issue.4, pp.475-491, 2002. ,
DOI : 10.1177/0049124102030004001